Boletín médico del Hospital Infantil de México

versión impresa ISSN 1665-1146

Resumen

Background. Scimitar syndrome is a rare congenital anomaly characterized by abnormal venous drainage of all or part of the right lung into the inferior vena cava due to partial systemic arterial supply, hypoplasia of the affected lung, or bronchial abnormalities and pulmonary lobulation. The fundamental change required is the right abnormal pulmonary venous return, total or upper two-thirds of the inferior vena cava (an image that resembles the scimitar or Turkish sword). The condition is diagnosed using laboratory analyses and clinical findings associated with chest x-ray. Case report. The radiographic image shows the presence of right heart, an image associated with decreased lung size. These findings require performing cardiac catheterization and/or angiotomography to confirm the diagnosis. The complication of this disease is in accordance with time of onset of symptoms of suspected short circuit from left to right. Signs of pulmonary hypertension are evidenced by echocardiography and the presence of congenital anomalies such as hypoplastic right lung, pulmonary sequestration, anomalous venous pressure and associated cardiovascular malformations. Conclusions. In older children and adults, the prognosis is good with conservative treatment. Surgical treatment is considered in symptomatic patients and in those patients with associated cardiac abnormalities.